Hereditary cerebellar ataxia (HA) are a heterogeneous group of disorders characterized by the presence of slowly progressive gait ataxia, dysarthria and other cerebellar signs. Detailed clinical history and neurologic as well as systemic examination are key to accurate diagnosis and hierarchical diagnostic investigations. The initial diagnostic evaluation of patients with HA should include a detailed assessment to rule out acquired treatable etiologies. If such a detailed screening is inconclusive, investigations should be directed towards hereditary causes of cerebellar ataxias. Early diagnosis and treatment can lead to favorable outcomes. Disease-specific therapies have emerged for various cerebellar ataxia syndromes including hereditary ones. Despite these advances, management of the majority of HA remains symptomatic. Moreover, there are no US FDA-approved medications for HAs to date. However, there are a few progressive HA conditions that can improve with disease-specific treatment, particularly if initiated early in the disease course. Here, we discuss various hereditary cerebellar ataxia syndromes that are amenable to disease-specific/targeted treatment.